Spina bifida

Stephen D MarkChristchurch Hospital

Christchurch

Disability not Inability

Major effect on Quality of Life

Expectations

Survival is a givenOptions available

Resources limitedSpecialized care

Community based

Objectives

• Incidence/prevention• Decision making• Multidisciplinary team• Urologic management• Future prospects….

Incidence/preventionReduced incidenceImproved nutritionFolate supplementTheraputic abortion

Early management

• Back closure: improved surgery reduced disability

• Multi disciplinary inputs….• Renal US: Kidneys, voiding function• Antibiotic prophylaxis

Goals:

Quality and Length of LifeRenal PreservationPrevent UTIContinence ( Bowels)Latex precautions

Decision making in Urologic therapy

• Timing and Type is controversial• Motivation and mobility• Support• IQ may be reduced• Hand function• Local expertise

Early vs Delayed

• Early• Ab Prophylaxis• Anti cholinergics• CIC• Renal US ( baseline)• MCU• UDS

• Manage to make bladderSafe (Pdet < 30 cm H20)Proactive treatment

–Late– Ab Prophylaxis– Renal US / MCU– Interval US

– CIC for emptying– Anticholinergics prn

– UDS prior to surgery

– Reactive treatment

WHAT I DO……….

Urologic management

Emptying: CIC ( Lapides 1970)Urethral / Mitrofanoff

Storage BladderAnticholinergicsBotoxAugmentation Outlet SlingInjectableAUSClosure BN

Note: Bowel management first

Bowel management

• In parallel with urinary tract• Regular complete emptying• Laxative• Enema

• ACE :

Issues:Fluid type and volumeCaecum vs Lt Colon

CIC : Outlet / Mitrofanoff

Lapides 1970’sSafe bladder emptyingUrethra is not sensateMitrofanoff in wheel chair patient

Complications rare… stricture, incontinence

Goal: Competent outlet that can be emptied

Bladder storage

• Goal: Low pressure high volume bladder• Voluntary voids: ( n=10/80)• Anticholinergics ( Oxybutinin ) ( n= 40/80 )• Botox ( n=1/80 )

• Ileal augmentation• Other bladder reconstruction

Bladder augmentation

34/802 died in F/U (UTI)

32 stable renal function1 re-augmentation

10 Mitrofanoff4 re-operation

Complications:

8 bladder calculi1 perforation1 laparotomy1 Latex allergy

Bladder Storage : Ileal augmentation

Ileal conduit : rare but still a place

Bladder storage: Outlet procedure

• Static resistance: Injectable, Sling ( skin ), reconstruction

Dynamic resistance: Artificial sphincter

N = 6 ( 1 bulbar )1 erosion : dry after removal1 malfunction4 success

Skin sling harvest

Bladder neck dissection + Bi valve bladder

Skin Sling sutured

Skin sling wrapped round bladder neck

Cinch bladder neck wrap

Skin sling outcomes

• 19 patients, 11 boys. Age mean 10 yrs• Follow up: 8 years, Sx, Renal US and UDS• 14/19 dry on CIC

• Durable outcome• No long term complications

My Preference

• Goal: Independence, QoL, Renal preservation, prevent UTI and Continent

• Early renal dilatation: CIC, Anticholinergics +/- augmentation

• Bowels OK, supportive situation. RUS follow up

• Incontinence stable kidney growth: CIC, Anticholinergics. Review… Persistant wetting skin sling and Ileal augmentation. Mitrofanoff if in wheelchair

Adolescent Transition

• Transfer chronic paediatric condition to adult care

• Independence• Complex psychological / physical issues• Different personnel, decision making

• Traumatic• Formal handover beneficial with local practitioners

Expensive: Wellington study

• NZMJ 2012, Mar 125 : Bowkett et al• Reviewed 6 consecutive spina bifida patients,

10 – 21 yrs.• 124 operations ( Av 20 )• 8.5 Days Av LOS ( 1066 total )• 7.5 CT scans• $ 944,000/ patient

Future Issues

• Reduced incidence• Antenatal intervention study ( MOMS ) 2018• Tissue engineering, Xiao procedure• Increased patient expectation• Adolescent transition/ Pregnancy, / Adult life• Long term follow up unknown ( Malignancy risk )

• Remember: ….a simple intervention can make a substantial difference…

Adam Hall : Gold medallist

Ref:1: Bowkett et al NZMJ 2012 Mar P 1252: Wilson RD et al J Obstet Gynaecol Can 2007 Mar 1933: Bauer J Urol 2011 14374: Mt Sainai J Med 2011 Sept 759